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Von Willebrand disease

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https://www.readbyqxmd.com/read/29230812/the-gut-microbiota-an-emerging-risk-factor-for-cardiovascular-and-cerebrovascular-disease
#1
REVIEW
Stefanie Ascher, Christoph Reinhardt
Commensal gut microbiota have recently been implicated in cardiovascular disease (CVD) and cerebrovascular disease. Atherosclerotic plaque formation depends on the colonization status of the host. In addition to host nutrition and the related microbiota-dependent metabolic changes, activation of innate immune pathways triggers the development of atherosclerosis and supports arterial thrombosis. Gnotobiotic mouse models have uncovered that activation of Toll-like receptor-2 by gut microbial ligands supports von Willebrand factor-integrin mediated platelet deposition to the site of vascular injury...
December 12, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/29227167/diagnosis-of-platelet-function-disorders-a-standardized-rational-and-modular-flow-cytometric-approach
#2
Oliver Andres, Katja Henning, Gabriele Strauß, Annerose Pflug, Georgi Manukjan, Harald Schulze
A high proportion of patients with mucocutaneous bleeding diathesis and suspected inherited or acquired platelet disorder remain without diagnosis even after comprehensive laboratory testing. Since flow cytometry allows investigation of resting and activated platelets on the single cell level by requiring only minimal amounts of blood, this method has become an important assay within the diagnostic algorithm, especially in pediatrics. We therefore developed a standardized and modular flow cytometric approach that contributes to clarify impaired platelet function in a rational step-by-step manner...
December 11, 2017: Platelets
https://www.readbyqxmd.com/read/29226875/vegfr1-and-vegfr2-in-alzheimer-s-disease
#3
Rachel Harris, James Scott Miners, Shelley Allen, Seth Love
Vascular endothelial growth factor (VEGF) is a potent angiogenic factor. Despite upregulation of VEGF in the brain in Alzheimer's disease (AD), probably in response to amyloid-β, vasoconstriction, and tissue hypoxia, there is no consequent increase in microvessel density. VEGF binds to and activates VEGF receptor 2 (VEGFR2), but also binds to VEGF receptor 1 (VEGFR1), which exists in less-active membrane-bound and inactive soluble (sVEGFR1) forms and inhibits pro-angiogenic signaling. We have investigated whether altered expression of VEGF receptors might account for the lack of angiogenic response to VEGF in AD...
December 6, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29225984/acquired-von-willebrand-disease-associated-with-monoclonal-gammopathy-of-unknown-significance
#4
Sijan Basnet, Catherine Lin, Rashmi Dhital, Izza Mir, Elan Mohanty, Biswaraj Tharu, Sushil Ghimire, Dilli Ram Poudel
We present a case of a 79-year-old male who presented with retroperitoneal hematoma a week after motor vehicle accident. Prior history and family history of bleeding were nonsignificant. His activated partial thromboplastin time was found to be prolonged in the emergency department. Further workup with coagulation studies showed decreased factor VIII, vWF antigen, and vWF:ristocetin cofactor assay, and negative Bethesda assay, indicating acquired von Willebrand disease. Immunofluorescence to find an underlying etiology was suggestive of MGUS...
2017: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/29222282/advances-in-the-diagnosis-and-treatment-of-von-willebrand-disease
#5
REVIEW
Ruchika Sharma, Veronica H Flood
Von Willebrand disease (VWD) is the most common inherited bleeding disorder, yet diagnosis and management remain challenging. Development and use of bleeding assessment tools allows for improved stratification of which patients may require further assessment and which patients are most likely to require treatment of their VWD. New options for laboratory assessment of von Willebrand factor (VWF) activity include a new platelet-binding assay, the VWF:GPIbM, which is subject to less variability than the ristocetin cofactor activity assay, and collagen-binding assays that provide insight into a different function of VWF...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29208651/a-12-3-kb-duplication-within-the-vwf-gene-in-pigs-affected-by-von-willebrand-disease-type-3
#6
Stefanie Lehner, Mahnaz Ekhlasi-Hundrieser, Carsten Detering, Hanna Allerkamp, Christiane Pfarrer, Mario von Depka Prondzinski
Von Willebrand Disease (VWD) type 3 is a serious and sometimes fatal hereditary bleeding disorder. In pigs, the disease has been known for decades and affected animals are used as models for the human disease. Due to the recessive mode of inheritance of VWD type 3, severe bleeding is typically seen in homozygous individuals. We sequenced the complete porcine VWF (Von Willebrand Factor) cDNA and detected a tandem duplication of exons 17 and 18, causing a frameshift and a premature termination codon (p.Val814LeufsTer3) in the affected pig...
December 5, 2017: G3: Genes—Genomes—Genetics
https://www.readbyqxmd.com/read/29208350/efficient-differentiation-of-bone-marrow-mesenchymal-stem-cells-into-endothelial-cells-in%C3%A2-vitro
#7
Chengen Wang, Yuan Li, Min Yang, Yinghua Zou, Huihui Liu, Zeyin Liang, Yue Yin, Guochen Niu, Ziguang Yan, Bihui Zhang
OBJECTIVE: Endothelial cells (ECs) play an important role in neovascularisation, but are too limited in number for adequate therapeutic applications. Mesenchymal stem cells (MSCs) have the potential to differentiate into endothelial lineage cells, which makes them attractive candidates for therapeutic angiogenesis. The aim of this study was to investigate efficient differentiation of MSCs into ECs by inducing medium in vitro. METHODS: MSCs were isolated from bone marrow by density gradient centrifugation...
December 2, 2017: European Journal of Vascular and Endovascular Surgery
https://www.readbyqxmd.com/read/29202604/characterization-of-von-willebrand-factor-multimer-structure-in-patients-with-severe-aortic-stenosis
#8
Joerg Kellermair, Helmut W Ott, Michael Spannagl, Josef Tomasits, Juergen Kammler, Hermann Blessberger, Christian Reiter, Clemens Steinwender
Acquired von Willebrand syndrome (AVWS) associated with severe aortic stenosis (AS) has been frequently subclassified into a subtype 2A based on the deficiency of high-molecular-weight (HMW) multimers as it is seen in inherited von Willebrand disease (VWD) type 2A. However, the multimeric phenotype of VWD type 2A does not only include an HMW deficiency but also a decrease in intermediate-molecular-weight (IMW) multimers and an abnormal inner triplet band pattern. These additional characteristics have not been evaluated in AVWS associated with severe AS...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29201071/hemostasis-in-overt-and-subclinical-hyperthyroidism
#9
REVIEW
Arash Ordookhani, Kenneth D Burman
Context: There are contradictory results on the effect of hyperthyroidism on hemostasis. Inadequate population-based studies limited their clinical implications, mainly on the risk of venous thromboembolism (VTE). The present review focuses on hemostatic changes in overt and subclinical hyperthyroidism. Methods: A systematic literature search was conducted employing MEDLINE database. The following words were used for the search: Hyperthyroidism; thyrotoxicosis; Graves disease; goiter, nodular; hemostasis; blood coagulation factors; blood coagulation disorders; venous thromboembolism; bleeding; fibrinolysis...
July 2017: International Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29200971/vwf-adamts13-is-associated-with-on-aspirin-residual-platelet-reactivity-and-clinical-outcome-in-patients-with-stable-coronary-artery-disease
#10
Ellen M K Warlo, Alf-Åge R Pettersen, Harald Arnesen, Ingebjørg Seljeflot
Background: The mechanisms behind residual platelet reactivity (RPR) despite aspirin treatment are not established. It has been shown that coronary artery disease (CAD) patients with high on-aspirin RPR have elevated levels of von Willebrand factor (vWF). ADAMTS13 is a metalloprotease cleaving ultra large vWF multimers into less active fragments.Our aim was to investigate whether ADAMTS13 and vWF/ADAMTS13 ratio were associated with high RPR, and further with clinical endpoints after 2 years...
2017: Thrombosis Journal
https://www.readbyqxmd.com/read/29198540/severity-and-features-of-epistaxis-in-children-with-a-mucocutaneous-bleeding-disorder
#11
Eva Stokhuijzen, Catherine I Segbefia, Tina T Biss, Dewi S Clark, Paula D James, Jim Riddel, Victor S Blanchette, Margaret L Rand
OBJECTIVE: To use standardized bleeding questionnaires to compare the severity and patterns of epistaxis in children with a mucocutaneous bleeding disorder and control children. STUDY DESIGN: The epistaxis sections of the Pediatric Bleeding Questionnaire (PBQ) administered to pediatric patients with von Willebrand disease or a platelet function disorder and healthy control children were reviewed. Scores and features of epistaxis (frequency, duration, onset, site, seasonal correlation, and need for medical/surgical intervention) were recorded...
November 30, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29194051/blood-tissue-and-imaging-biomarkers-in-calcific-aortic-valve-stenosis-past-present-and-future
#12
Mylène Shen, Lionel Tastet, Jutta Bergler-Klein, Philippe Pibarot, Marie-Annick Clavel
PURPOSE OF REVIEW: Calcific aortic valve stenosis is the most prevalent valvular heart disease in the high-income countries. To this date, no medical therapy has been proven to prevent or to stop the progression of aortic valve stenosis. The physiopathology of aortic valve stenosis is highly complex and involves several signalling pathways, as well as genetic related factors, which delay the elaboration of effective pharmacotherapies. Moreover, it is difficult to predict accurately the progression of the valve stenosis and finding the optimal timing for aortic valve replacement remains challenging...
November 30, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/29187375/advances-in-the-diagnosis-and-treatment-of-von-willebrand-disease
#13
REVIEW
Ruchika Sharma, Veronica H Flood
Von Willebrand disease (VWD) is the most common inherited bleeding disorder, yet diagnosis and management remain challenging. Development and use of bleeding assessment tools allows for improved stratification of which patients may require further assessment and which patients are most likely to require treatment of their VWD. New options for laboratory assessment of von Willebrand factor (VWF) activity include a new platelet-binding assay, the VWF:GPIbM, which is subject to less variability than the ristocetin cofactor activity assay, and collagen-binding assays that provide insight into a different function of VWF...
November 30, 2017: Blood
https://www.readbyqxmd.com/read/29186156/a-common-mechanism-by-which-type-2a-von-willebrand-disease-mutations-enhance-adamts13-proteolysis-revealed-with-a-von-willebrand-factor-a2-domain-fret-construct
#14
Christopher J Lynch, Adam D Cawte, Carolyn M Millar, David Rueda, David A Lane
Rheological forces in the blood trigger the unfolding of von Willebrand factor (VWF) and its A2 domain, exposing the scissile bond for proteolysis by ADAMTS13. Under quiescent conditions, the scissile bond is hidden by the folded structure due to the stabilisation provided by the structural specialisations of the VWF A2 domain, a vicinal disulphide bond, a calcium binding site and a N1574-glycan.The reduced circulating high MW multimers of VWF in patients with type 2A von Willebrand disease (VWD) may be associated with mutations within the VWF A2 domain and this is attributed to enhanced ADAMTS13 proteolysis...
2017: PloS One
https://www.readbyqxmd.com/read/29168609/the-increasing-maturity-of-the-von-willebrand-factor-collagen-binding-in-von-willebrand-disease-diagnosis
#15
E J Favaloro, S Mohammed, S Oliver
No abstract text is available yet for this article.
November 23, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29168270/a-comparative-evaluation-of-a-new-fully-automated-assay-for-von-willebrand-factor-collagen-binding-activity-to-an-established-method
#16
F Stufano, L Baronciani, D Mane-Padros, G Cozzi, S Faraudo, F Peyvandi
INTRODUCTION: Laboratory diagnosis of von Willebrand disease (VWD) is made by the measurement of von Willebrand factor (VWF) protein level and its activities. Current VWF activity tests include ristocetin cofactor and collagen binding (VWF:CB) assays. AIM: We have undertaken an evaluation of a new fully automated VWF:CB assay relative to an established enzyme-linked immunosorbent assay (ELISA) method. METHODS: The two analytical systems operate with different detection principles: a chemiluminescent method performed on ACL AcuStar Analyzer (the former) and a colorimetric ELISA by Asserachrom Stago (the latter) (type III collagen from human placenta)...
November 22, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29167411/2-chlorofatty-acids-induce-weibel-palade-body-mobilization
#17
Celine L Hartman, Mark A Duerr, Carolyn J Albert, William L Neumann, Jane McHowat, David A Ford
Endothelial dysfunction is a hallmark of multiple inflammatory diseases. Leukocyte interactions with the endothelium have significant effects on vascular wall biology and pathophysiology. Myeloperoxidase (MPO)-derived oxidant products released from leukocytes are potential mediators of inflammation and endothelial dysfunction. 2-Chlorofatty acids (2-ClFAs) are produced as a result of MPO-derived HOCl targeting plasmalogen phospholipids. Chlorinated lipids have been shown to be associated with multiple inflammatory diseases, but their impact on surrounding endothelial cells has not been examined...
November 22, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/29166863/characterization-of-the-inner-membrane-protein-bb0173-from-borrelia-burgdorferi
#18
Christina M Brock, Manuel Bañó-Polo, Maria J Garcia-Murria, Ismael Mingarro, Maria Esteve-Gasent
BACKGROUND: The bacterial spirochete Borrelia burgdorferi is the causative agent of the most commonly reported arthropod-borne illness in the United States, Lyme disease. A family of proteins containing von Willebrand Factor A (VWFA) domains adjacent to a MoxR AAA+ ATPase have been found to be highly conserved in the genus Borrelia. Previously, a VWFA domain containing protein of B. burgdorferi, BB0172, was determined to be an outer membrane protein capable of binding integrin α3β1...
November 22, 2017: BMC Microbiology
https://www.readbyqxmd.com/read/29165741/emerging-roles-for-von-willebrand-factor-in-cancer-cell-biology
#19
Jamie M O'Sullivan, Roger J S Preston, Tracy Robson, James S O'Donnell
von Willebrand factor (VWF) is a complex multimeric plasma glycoprotein that plays critical roles in normal hemostasis. However, additional novel roles for VWF in modulating cancer cell biology, and in particular tumor metastasis, have recently been reported. Markedly elevated plasma VWF levels were associated with advanced tumor stage and metastatic disease. These observations have raised the question of whether VWF may be involved in regulating tumor progression. Interestingly, novel findings indicate that VWF is expressed by a variety of tumor cells of nonendothelial origin...
November 17, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29165738/alloantibodies-in-von-willebrand-disease
#20
Massimo Franchini, Pier Mannuccio Mannucci
von Willebrand disease (VWD), the most commonly known inherited bleeding disorder, is caused by a partial (type 1) or total (type 3) deficiency or dysfunction (type 2) of von Willebrand factor (VWF). Its management encompasses the prevention or treatment of bleeding by raising endogenous VWF levels using a synthetic agent, such as desmopressin, or providing exogenous VWF concentrates. The development of inhibitory alloantibodies against VWF is a rare but often severe complication encountered during the treatment of type 3 VWD, which is associated with a lack of hemostatic response to infused VWF concentrates and more rarely with allergic, even anaphylactic, reactions...
November 17, 2017: Seminars in Thrombosis and Hemostasis
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